Benjamin "Benjy" Stacy so frightened maternity doctors with the color of his skin -- "as Blue as Lake Louise" -- that he was rushed just hours after his birth in 1975 to University of Kentucky Medical Center.Turns out the family has a recessive genetic condition known as Methemoglobinemia. This causes the body to produce excess methemoglobin, a faulty form of hemoglobin that doesn't carry oxygen. This tinges the blood blue, giving sufferers purple lips and bluish skin. The variant carried by this family seems to cause no serious damage, since many of the bluest lived into their 80s or 90s.
As a transfusion was being readied, the baby's grandmother suggested to doctors that he looked like the "blue Fugates of Troublesome Creek." Relatives described the boy's great-grandmother Luna Fugate as "blue all over," and "the bluest woman I ever saw."
In an unusual story that involves both genetics and geography, an entire family from isolated Appalachia was tinged blue. Their ancestral line began six generations earlier with a French orphan, Martin Fugate, who settled in Eastern Kentucky.
And now for your dose of Kentucky stereotyping:
The Fugate progeny had a genetic condition called methemoglobinemia, which was passed down through a recessive gene and blossomed through intermarriage. . . . the community remained small and isolated. The Fugates married other Fugate cousins and families who lived nearby, with names like Combs, Smith, Ritchie and Stacy. Benjy's father, Alva Stacy showed Trost his family tree and remarked, "If you'll notice -- I'm kin to myself."I keep hearing a voice saying, "And the Lord cursed them for their sin, and put the mark of Fugate upon them, so that their skin was always blue. . . ." And how perfect is it that they live on Troublesome Creek?